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154 Cards in this Set

  • Front
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OA is Degeneration of this
degenerative changes of articular cartilage
These cells play a role in OA
Marginal osteophytes
What age is most affected by OA
Age > 40 years
> 80% over 75 years old
Female > Male
In those >65 years, symptomatic 11% knee, 5% hip
Primary Cause of OA is
Majority is Idiopathic
What are the secondary causes of OA
Congential disorders
Metabolic disorders-Hemachromatosis, Crystal deposition diseases
Apart from the primary and secondary causes of OA what are the other causes?
Post-Traumatic and Post-Surgical OA
Congential disorders that can cause OA
SCFE, congenital hip dislocation
Mechanical features
leg-length discrepancy
Varus/valus deformity
Scoliosis
W are the risk factors for OA
Obesity – knees
Occupational – bending, carrying heavy loads
Sport activity
Heredity – DIPs
What clinical features would be seen in OA
Slow onset
Morning stiffness less than 30 minutes, if present
Gelling
Pain in involved joint
Bony joint enlargement
Crepitation
Effusion possible
Typically no soft tissue swelling
Is there any diagnostic lab for OA
No, synovial fluid evaluation can differentiate nature of effusion
What would U see on an X-ray for OA
X-Ray evaluations
Bony spurs – osteophytes
Subchondral sclerosis, Subchondral cysts
Joint space narrowing –
Knees - Consider weight bearing view
Tx for OA can include
Conservative Tx: Regular exercise/muscle strengthening, Weight loss
Physical therapy,
Splinting- CMC spica, supportive braces and unloading
Pt’s OA Sx can be relieved w/
Symptomatic relief: Topicals OTC
Hot/cold creams – menthol based
Capsaicin, Methyl salicylate, Paraffin baths for hand OA
What's the 1st line and 2nd line tx for OA
First line: Acetominophen
Second line- Oral NSAIDs,
Topicals -prescription
Gels, patches
Tramadol can be used in OA when?
As a non-opiate medication option, eg tramadol
Other tx, besides NSAIDS and Tramadol, for OA include
Local treatments:
Intra-articular Injection, Corticosteroids, Viscosupplementation
Opiates
Surgery
What is the indication for Viscosupplementation in OA
Indication:
Relief from/ to avoid NSAID use
Inadequate response to NSAIDs and topicals, and injections
Delay surgery
These two meds can be used to treat individual joints like the hip, and knees in OA
Hyaluronan and hylan (HA)
Knees, some recent study of efficacy in the hip
Typically have longer effect versus IA steroids
What caution should be noted with using Hyaluronan and hylan for OA?
Allergy to birds, feathers, and eggs
What are the risk factors for RA
Genetics, Smoking and Other environmental factors
rheumatoid arthritis is diagnosed when..
Morning stiffness lasting at least 1 hr
2. Soft- tissue swelling or fluid in at least 3 joint areas simultaneously
3. At least one area swollen in a wrist, MCP, or PIP joint
4. Symmetric arthritis
5. Rheumatoid nodules
6. Abnormal amounts of serum rheumatoid factor
7. Erosions or bony decalcification on radiographs of the hand and wrist
least 4 of these 7 criteria are present, and criteria 1 to 4 are present at least 6 weeks
How does RA manifest in the hands?
Second and third MCP synovial thickening /synovitis
Pain on squeeze of the MCPs
Secondary carpal tunnel, ‘boxers gloves’
Triggering secondary to tenosynovitis
Decreased grip
Boutonniere's and swan neck deformities
Ulnar deviation
MCP subluxation
Other manifestions of RA (apart from the hands) include
Profound fatigue
Sleep disturbances secondary to pain
Early may not be symmetric
Occasionally recurrent oligoarthritis
Palindromic – relapsing/remitting periods of stiffness/joint pain
May present with joint pain, periodic low grade fevers
X-ray of your RA hand would show
Periarticular soft-tissue swelling
Juxta-articular osteopenia
Marginal erosions
Joint-space narrowing
Symmetric involvement
Deformities in advanced disease
C1-C2 atlantoaxial subluxation in RA is treated how?
Immobilization collar

Contraindicated procedures/therapy
Doing Surgery – fusion
Prevents odontoid migration, Cord compression
Presence of swan-neck and boutonnière deformity indicates
Indicates RA
The progression of RA depends on
Disease state at time of diagnosis
Ability to induce remission
Presence of Anti – Cyclic Citrullinated peptide antibody
What are other Non articular related risks/disease associated with RA
cardiovascular disease
Infection
Pneumonitis/ interstitial lung disease
Lymphoma
Overall decreased life expectancy dependent upon disease progression and control
Preliminary evaluation for presence of RA should include
CBC, LFT CRP, ESR BUN,CRE, Rheumatoid Factor, Anti-cyclic citrullinated peptide (CCP) Antibodies
What role does the rheumatic factor play
IgM Antibody to the Fc portion of IgG
Higher levels associated progressive disease
Present in approximately 80% of RA patients
May be negative in early disease, or in some RA patients
May be present in other disorders, both rheumatic and non-rheumatic disease
High association with Rheumatoid Arthritis
Indicates a more aggressive course
Associated with peri-articular erosions
Anti –CCP antibody
Methotrexate (MTX) is a mainstay of treatment
If MTX is insufficient, use combination treatment
If failure persists on combination treatment, consider adding a TNF-Alpha inhibitor
Tx for RA
Disease Modifying Anti-Rheumatic Drugs (DMARDs) for TX of RA include?
Corticosteroids, Methotrexate , Hydroxychloroquine, Leflunomide
TNF-Alpha inhibitors
Other therapies
B-Cell related therapy, Rituximab
T cell related therapy, Abatacept
IL – 6 inhibitor, Tocilizumab
Used in patients who have failed one or more TNF-alpha inhibitors
May be used with or without another DMARD, eg methotrexate
Tocilizumab
RA – Remission ACR criteria
minimum of five of the following for at least 2 consecutive months Morning stiffness not to exceed 15 minutes
No fatigue
No joint pain
No joint tenderness or pain on motion
No soft tissue swelling in joints or tendon sheaths
ESR (Westergren's method) less than 30mm/hour (females) or 20mm/hour (males
Disease onset <16 years of age
Persistent arthritis 1 or > joints for 6/+ weeks
Occurs in absence of other types of arthritis or diseases
Juvenile Idiopathic Arthritis (JIA)
This is a subtype of JIA - Mild, may have no swelling, non-systemic
Pauciarticular
Complications of JIA include
Uveitis -May be silent until have vision loss
Frequent eye exams and More common with pos ANA
Leg length discrepancies
Tx for JIA includes
Treatment – NSAIDs, IA injection
Exclude joint infection prior to CS injections
DMARDs less likely
May start similar to pauciarticular, then progress to 5+ joints
Often symmetric knees, wrists, ankles
Polyarticular JIA
This is seen as a complication of Poly Articular JIA
Dactylitis can be present
Systemic JIA manifests with?
Quotidian fever
Evanescent/Salmon-colored rash
Ill with fever, may appear well when defervesce
Koebner phenomenon
Arthralgias > arthritis (may present later)
What is the TX for JIA's?
DMARDS
Quotidian fever can be seen in which type of JIA
Systemic JIA
With this JIA The kids are sick! And should r/o infection/hematologic disease
Systemic JIA
Labs for Systemic JIA would show
markedly elevated WBC, Elevated platelets, LFTs and Ferritin, ESR, Anemia

Imaging should also be done
Important considerations in Tx of JIA
Active Inflammatory disease = skeletal damage
May be irreversible if long term
Early aggressive treatment needed to quickly control SX/prevent damage
RF and Anti-CCP ab are often negative
Anterior uveitis frequent monitoring imperative \
Psychosocial issues
Tissue deposition of monosodium urate crystals due to hyperuricemicia
Gout
What are the manifestations of gout?
Acute attack
Gouty arthritis
Tophi
Uric acid nephrolithiasis
Hyperuricemia develops due to
Overproduction of urate (10%)
Ethanol - beer
dietary purine precursors
G6PD deficiency
Myeloproliferative disorders
Drugs contributing to hyperuricemia
Due to decreased renal excretion of urate
Alcohol
Loop diuretics – furosemide
Thiazide diuretics
Low dose aspirin
Foods that trigger gout attack
Purine rich foods
Meats – especially organs
Seafood, especially shellfish, sardines, anchovies
Vegetables/legumes – asparagus, cauliflower, spinach, beans, peas, mushrooms
What crystals are involved with a gout attack
Crystals are coated with IgG
Attack triggers for gout
Acute medical illness, e.g. infections
Trauma
Surgery
Dehydration/rehydration
Clinical symptoms for Gout includes
Pain, pain, pain
erythema, swelling
often lower extremity, 1st MTP or knee
Peak pain in hours
Self limited - spontaneous resolution days to weeks
May involve multiple joints
Gout can be Self limited - spontaneous resolution days to weeks T/F
True
Labs for Gout include what orders
Uric acid level: uric acid levels fall during acute attack, Don’t be fooled!
Uric acid level during attack can be in WNL!
24 hour urine uric acid test
Evaluation of fluid aspirate
X-rays
Can be seen on X-rays in gout
Soft tissue swelling
Tophi
Bony erosions “rat bite erosions
X- ray for gout show what
Soft tissue swelling
Tophi
Bony erosions “rat bite erosions
24 hour urine uric acid test with gout shows what?
Underexcretion - <800 mg/24h
Overproduction - >800 mg/24h
Gout – polarized light microscopy will show what with the crystals?
Blue in perpendicular and yellow in parallel light
Uric Acid levels can be low during a gout attack T/F
True
What are the indications for tx of Gout
>2-3 attacks within 1-2 years
Renal stones (Ca or urate)
Tophaceous gout
Often fingers, olecranon, also larynx, kidney, heart valve, anywhere…..
Chronic gouty arthritis with erosions
Asymptomatic hyperuricemia?
>12 mg/dL or 24 hour urine excretion >1100mg
Some rheum recc rx >10…..
Hyperuricemia - CV risk
Tx of gout
NSAIDs

Glucocorticoids e.g Prednisone: caution – DM

Colchicine: New recommendation : Avoid q hour dosing
Require decrease dose in renal insufficiency
NSAIDs used in gout Tx
Reversibly inhibits cyclooxygenase
Strong anti-inflammatory
Indomethacin
Side effects for indomethacin in gout TX
N/V/D, anorexia, abdominal pain
Frontal headaches, dizziness, Neutropenia, thrombocytopenia
Indomethacin, TX for gout, is contraindicated in
third trimester pregnancy / Category B, not for 3rd trimester or neonates
Black box: CV – increase thrombotic events, MI, Stroke; ulceration/perforation/hemorrhage
What can be used for long term tx of Gout
Colchicine

Xanthine oxidase inhibitors/overproduction
1.Allopurinol
2.Fubuxostat – recent approval

Uricosurics /underexcretion
1.Probenecid
2.sufinpyrazone
Side effects of Allopurinol, TX of gout?
Alopecia
Leukopenia
GI symptoms
Increase risk of attack at initiation/adjustment
Uricosurics
1.Probenecid
2.sufinpyrazone

TX for Gout causes what side effects?
Major side effects: rash, GI irritation, hypersensitivity, precipitation of acute attack, and kidney stone formation
This is asymptomatic or symptomatic

Aka chondrocalcinosis (radiographic)
CPPD = Calcium Pyrophosphate Deposition Disease
symptomatic deposition of CPPD in a joint with a subsequent inflammatory response
acute attack May clinically appear similar to Gout
Pseudogout
Causes of CPPD
Idiopathic – majority
Joint trauma
Familial – ankh gene mutation effecting pyrophosphate transit channels
Aberrant Enzyme NTPPPH, pyrophosphate overproduction
Associated diseases that cause Gout
Hemochromatosis
Hyperparathyroidism
Hypophosphatasia
Hypomagnesemia
Gitleman's syndrome – inherited renal tubular d/o
Clinical features of gout
Typically effects the knee, larger joints
Self limited
Pain, stiffness, swelling of the affected joint
Systemic manifestation of gout include
Fever, leukocytosis, ESR elevation.
What can be used in the diagnosis of CPPD
Polarized light microscopy of fluid aspirate
Cell count
X-Ray
Ultrasound
Seen on polarized light for CPPD
rhomboid crystals
Synovial fluid analysis shows what for CPPD vs OA
CPPD - Inflammation, Cloudy, yellow
2000-100,000 leukocyte count, decreased viscosity

OA- Non inflammatory, clear, yellow and viscous. 200-2000 leukocyte count
Responds to steroid tx in CPPD
Chondrocalcinosis – Knee
How do you TX Acute pseudogout
rest joint, consider splinting to protect
1-2 joints - joint aspiration, and IA steroid injection
Multiple joints tx in PGout can be tx using
Oral prednisone, NSAIDs, colchicine
Chronic (>2 attacks per year) pseudo gout is treated how?
Colchicine, or if contraindicated, NSAIDs

Joint degeneration is treated as OA
This is a chronic inflammatory skin disease. The different types include plaque, guttae, pustular, and erythrodermic. May be associated with inflammatory arthritis.
Psoriasis
This manifests with morning pain and stiffness over 30 minutes. Asymmetric arthritis, oligoarticular-DIPs, Enthesitis (inflammation where joints insert), Dactylitis - "sausage digits".
Psoriatic Arthritis
The clinical manifestations of this show nail changes - pitting, onchyolysis, transverse ridging. Anteriior uveitis, "Rheumatoid like" disease, other forms can show up with this such as Arthritis mutilans, axial involvement such as poor internal rotation of hips - Asymmetric sacroillitis, and axial syndesmophytes.
Psoriatic Arthritis
If you see inflammatory arthritis and a red eye, what can this mean?
Anterior uveitis which needs an immediate ophthalmology consult.
What is involved with the axial involvement assocaited with psoriatic arthritis?
Sacroillitis

Lumbar Spine - Syndesmocytes
What diagnostic studies do you order for psoriatic arthritis?
CBC
ESR, CRP
Rheumatoid factor, Anti-CCP antibody
NB uric acid
Cre, others in consideration of therapy
What will be seen on X-Ray imaging with psoriatic arthritis?
"pencil and cup" appearance of interphalangeal joint.

Erosions

Fluffy periosteal bone formation

Enthesopathy

Sacroilitis

Syndesmophytes - asymmetric/coarse
What is the TX for psoriatic arthritis?
NSAIDS

Prednisone

Sulfasalazine

Methotrexate - don't use with patient who drinks

Leflunomide - don't use with patient who drinks

TNF-Alpha inhibitors
What DMARDS are used to TX psoriatic arthritis?
Prednisone

Methotrexate - Unsafe/contraindicated for lactation, avoid in patients who drink

Sulfasalzine - inhibit prostaglandin synthesis, contraindicated third trimester/lactation safety unknown/avoid, avoid with G6PD deficiency, sulfonamide or salicylate allergy

Leflunomide - category X, lactation unsafe, pregnancy should not be attempted for two years after discontinuation

TNF-Alpha Inhib. - Etanercept, Infliximab, Golimumab, Adalimumab, inhibition of TNF-A leads to neutralization without cell destruction.
What are the major side effects with TNF-Alpha Inhibitors?
Serious infection
Reactivation of latent TB
Reactivation of Hep B
Worsening of heart failure
This is a non-purulent inflammatory arthritis occurring following infection. Symptoms follow typically a GI or GU infection. Reactive/Reiter's triad shows with this = Arthritis, Urethritis, Conjunctivitis.
Reactive Arthritis
The following infections are associated with what?

Bacteria:
GI - Salmonella, Yersinia, Shigella, Camphylobacter, Clostridium difficile.

GU - Chlamydia, Gonorrhea
Reactive Arthritis
This occurs approximately 1-4 weeks following gastrintestinal or sexually transmitted infection. While infection may clear quickly, arthritis may persist for months or become chronic.
Reactive Arthritis
This presents with Asymmetric oligoarthritis, Enthesitis - especially in achilles, Dactylitis.
Reactive Arthritis
With this you can see inflammatory eye disease - conjunctivitis, anterior uveitis

Mucocutaneous lesions - Balantitis, Stomatitis, Keratoderma blennorhagicum

Joint presentations - synovitis/effusion, enthesitis
Reactive Arthritis
Keratoderma blenorrhagicum associated with reactive arthritis can show up with a patient who has what disease?
HIV
What laboratory evaluations are associated with reactive arthritis?
Evidence of GI/GU infection via culture (not necessary for diagnosis, but for rx)

Aspiration effusion w/ monoarthritis
Fluid studies
Cell count, crystals, culture, glucose, consider PCR lyme, chlamydia

Urine chlamydia, stool culture
What is the TX for reactive Arthritis?
Treat infection if present
Arthritis may spontaneously remit after 6 months
Small percentage persist
Treatment is dependent upon severity
NSAIDS, local injection
If conservative fails/doesn’t resolve, then DMARDs
Inflammatory muscle disease
Effects striated muscle
Occasionally effects cardiac muscle

Can occur with Dermatomyositis
Cutaneous involvement
Heliotrophe rash, gottron’s papules, shawl (or “V”) sign
calcinosis
Can be associated with underlying malignancy
Polymyositis
Symmetric proximal muscle weakness
Occurs slowly over months
Prominent shoulder/pelvic girdle/neck flexors

Symmetric proximal muscle pain accompanying weakness

May have cardiac or pulmonary involvement
Conduction defects
ILD

May have GI involvement
dysphagia/regurg/aspiration

Respiratory
Diaphragm/chest wall muscle involvement
Polymyositis
What is involved with the evaluation of polymyositis?
Elevated muscle enzymes
CK
Others – aldolase, myoglobin, AST/ALT, LDH, myoglobinuria

EMG changes
Myopathic

Muscle biopsy
Obtained from weak muscle
Usually quad or deltoid
Peri-vascular and endomyosial inflammation with fiber necrosis and degeneration
These may occur with Dermatomyosis

MRI - T2 imaging (fat suppression) for appropriate site guidance
What is the TX for polymyositis?
Glucocorticoids

High dose initially with goal to taper over 9 months to a year to minimize

May require long term medication to spare steroid

Azathioprine
Check TPMT, thiopurinemethyltranferase - enzyme

Methotrexate
What do you do to monitor polymyositis?
Treatment associated side effects

Age related cancer screenings

Chest-Xrays - Interstitial lung disease risk
A systemic necrotizing vasculitis effecting small and medium sized arteries, that can lead to secondary tissue/organ ischemia.
Polyarteritis Nodosa - Very Rare
This is associated with hepatitis B
Polyarteritis Nodosa
The following presentation is associated with what?

Constitutional symptoms
Fatigue, weight loss, tachycardia, fever, myalgias, arthralgias
Skin
Livedo reticularis, nodules, digital ischemia, ulcerations
Peripheral nerves
Mononeuritis multiplex
GI/GU
Intestinal angina
Mesenteric infarctions or aneurysms
Testicular pain
Renal
Renal and interlobular arteries
Microaneurysms, wedge shaped renal infarcts
Renin-related hypertension
Cardiac
Patchy necrosis of myocardium
Tachycardia – reflects direct damage or inflammatory state
Polyarteritis Nodosa
What is the TX for Polyarteritis Nodosa?
Glucocorticoids

Localized or systemic
Local - Cytotoxic agents –if steroid unsuccessful/inadequate
Systemic – Cyclophosphamide

Treat Hepatitis B if present
What is the side effects of cyclophosphamide, aka cytoxan when given for polyarteritis nodosa TX?
Hemorrhagic cystitis

Sterility

CI: live vaccines due to immunosuppression

Category D; fetal risk, unsafe lactation
Can be a "one time" disease where 80% of patients will go into remission after two years.
Polyarteritis nodosa
A constellation of symptoms that may include diffuse body pain, sleep disturbance, and depression

“the invisible syndrome”
Fibromyalgia
The following are associated with what?

"central sensitization" - change in pain perception

Abnormalities in sensory processing

Stressors – physical/psychiatric trauma

Sleep disturbances – apnea, restless legs, obesity

Polypharmacy
Fibromyalgia
The following symptoms are associated with?

General fatigue
Anxiety
Increased pain in morning, or with anxiety or stress
Chronic headaches
c/o alternating diarrhea/constipation
Subjective swelling or numbness w/o associated objective findings
Non-restorative sleep
Fibromyalgia
Pain in 11 of 18 tender point sites on digital palpation with approximate force of 4kg. This is associated with what?
ACR 1990 Classification Criteria: Fibromyalgia
How is fibromyalgia diagnosed?
Diagnosis of exclusion - Look for underlying causes
What are the TXs for fibromyalgia?
Must treat co-morbid depression/anxiety if present

Maintain activity

Treat sleep disturbances

Treat depression
What is the pharmacotherapy used for fibromyalgia?
Tricyclic Antidepressants : Cyclobenzaprine (Flexeril), Amitriptyline (Elavil)

SNRI: Duloxitine (Cymbalta)

Anticonvulsant: Pregabalin (Lyrica)
Rheumatic disease characterized by acute or sub-acute onset pain and stiffness of the proximal neck, shoulder girdle, and pelvic girdle of at least four weeks duration
Polymyalgia Rheumatica (PMR)
More common in Northern European, Scandinavian descent
Associated with Giant Cell Arteritis (GCA)
Polymyagia Rheumatica (PMR)
Primarily a clinical diagnosis
Acute or sub-acute onset
Proximal pain in shoulders, lower extremities, typically symmetric
Stiffness of shoulders, hips
Absence of objective muscle weakness
Exclude physical cause

Laboratory testing
CBC, ESR, CRP
PMR Diagnosis
How do you TX PMR?
Prednisone
15-20 mg daily

With control of symptoms, commence taper

Self-limited, and may be able to taper off completely after 1-2 years
How do you monitor PMR?
ESR, CRP, CBC prior to each visit
Monitor for side effects of prednisone
Monitor for recurrence of sx as taper
May require secondary agent if unable to wean off prenisone without recurrence.
What are the signs and symptoms of giant cell arteritis?
Signs and symptoms of GCA
Amaurosis fugax / vision changes
Jaw claudication
Headaches – temporal or other
Fever
PMR
This is a chronic systemic autoimmune disorder effecting most major organ systems.
Systemic Lupus Erythematosus
The pathogenesis of this disease is that it develops auto antibodies causing cell destruction/apoptosis.
Systemic Lupus Erythematosus
What is the main complaint a patient has with SLE?
Being tired
The general symptoms associated with this are as follows:

Fatigue
Fever
Weight loss
Myalgias/Arthralgias
Alopecia
Rashes
Lymphadenopathy
SLE
What manifestations are seen with SLE?
Malar/Butterfly Rash
Interarticular dermatitis
Stomatitis
Jaccoud's Arthropathy
What pulmonary findings are associated with SLE?
Acute lupus pneumonitis - fever, cough, dyspnea, hypoxia, basilar rales, pleural effusion, pulmonary infiltrates on X-Ray, no apparent infection, e.g. negative culture.

Pleural effusions

Interstitial lung disease
What is associated with the renal involvement in SLE?
Glomerulonephritis - monitor urinalysis for proteinuria and monitor creatinine/GFR
What are the complications associated with SLE?
Major cause of death-accelerated atherosclerosis, CV

Renal failure in lupus nephritis

Strokes - with positive APL syndrome

Opportunistic infections

Malignancies - higher risk of cervical dysplasia and carcinoma in females
What is the TX for SLE?
Conservative:

Skin - At least SPF 30 sunscreen, topical gluticocorticoids, systemic glucocorticoids

Smoking Cessation - Increased risk of flare

Treat Hyperlipidemia

Avoid Sulfonamides

Avoid high dose estrogen
What are the medications used in SLE systemic therapies?
Typical Medications - Glucocorticoids, Hydroxychloroquine, Azathioprine
What immunosuppressive medications do you use in SLE - Systemic treatments/organ involvement?
Methotrexate - Arthritis unresponsive to NSAIDS

Azathioprine/Mycophenolate - Mild nephritis, or in remission after pulsed cyclophosphamide

Cyclophosphamide - Severe lupus nephritis
This is an episodic, reversible digital skin color change due to vasospasm

Usually cold induced, sometimes emotional distress
Raynaud's Phenomenon
This manifests as the following?

Biphasic color changes with cold exposure

Symmetric attacks

No digital ulcers, pits, or gangrene

No PVD

Normal nail fold capillary examination

Negative ANA and ESR
Primary Raynaud's Phenomenon
How do you TX Raynaud's phenomenon?
Exclude underlying cause

Conservative - Gloves, mittens, warm clothing, avoid cold exposure, avoid exacerbating medications/substances - avoid caffeine, amphetamine, decongestants (sympathomimetics), smoking cessation

Medications - no FDA approved TX, but CCB, Indirect Vasodilator-fluoxetine, phoshodiesterase inhibitor-sildenafil
If you see Raynaud's with late onset over 30 years old, male, ulcerations, pitting, involvement of more than distal digits, involvement of the feet, nail fold changes, laboratory testing abnormalities - ANA, RF
Secondary cause of Raynaud's
This is a small vessel vasculopathy that effects small arteries, arterioles, and capillaries causing ischemic reperfusion injury. Proliferation of cells in the intima of small vessels, leading to cascade causing obliteration and proliferative changes - digital ischemia/ulceration, pulmonary hypertension. Increased sensitivity to cold and stress - Raynaud's in 95% of patients.

Fibrosis - Activation of fibroblasts with increased production of collagen, leading to skin thickening
Systemic Sclerosis
What are the early manifestations of Systemic Sclerosis?
Raynaud's phenomenon

GERD

Fatigue

Musculoskeletal complaints
Systemic sclerosis is put into what two categories?
Limited and Diffuse

Limited - limited to hands, feet and face

Diffuse - throughout body, not limited to hands, face and feet
This is a dermatologic presentation of systemic sclerosis seen on the body?
En coup de sabre
What diagnostic testing do you use for systemic sclerosis?
Anti-centromere antibody

Anti-SCL-70 antibody

Nail fold capillary examination
What are the following?

Toxin induced skin changes -
vinyl chloride
bleomycin
organic solvents and resins

Vibration injury

Eosinophilic fasciitis - cobblestone like skin
Scleroderma-Like syndromes
What are the following involved with?

Interstitial fibrosis

Pulmonary hypertension

Scleroderma renal crisis

Digital ulceration/ischemia
Sequellae of systemic sclerosis
How do you treat systemic sclerosis?
No proven disease modifying medication

Treat Raynaud's

Watchful waiting/monitoring

Treatment of other individual manifestations:
Digital ulcers
Renal crisis
Interstitial lung disease
PAH
GI disease
What is involved with monitoring systemic sclerosis?
Pulmonary function tests at baseline and consider yearly or with change in symptoms.

Echocardiogram at baseline and then annually

Chest X-Ray at baseline and then annually

BP monitoring

Creatinine, Urinalysis

Skin Integrity
This is an autoimmune disease involving salivary, lacrimal and exocrine glands. Glands infiltrated by CD4+ T cells, and some B cells. Infiltration by inflammatory cytokines
Sjogren's Syndrome
Inflammatory state leads to secretory duct and acinar cell damage. Parotid luminal narrowing, keratoconjunctivitis sicca, GI mucosal dryness and atrophy-dysphagia, vaginal dryness.
Sjogren's Syndrome
What is the clinical presentation of Sjogren's Syndrome?
Dry eyes - irritation, photosensitivity, later corneal damage

Dry mouth - tooth decay, candida infections, salivary stones, difficulty swallowing

Respiratory tract - dry cough

Vaginal mucosa - candida infections
How do you diagnose sjogrens?
Positive eye tests - Schirmer's test, Rose Bengal Stain, Slit-Lamp examination-Fluorescein

Auto-antibodies - Anti Ro (SS-A), Anti La (SS-B), May have positive rheumatoid factor, may have elevation of ESR
What is the TX for sjogrens?
Conservative:

Regular ophthalmic and dental care

Lubricants - Artificial tears/ocular lubricants-hypomellose, methycellulose, gels at night

Punctual occlusion

Avoid exacerbating issues, eg. contact lenses (soft lenses), and LASIX (contraindicated)

Oral - sialogogues - lemon drops, sugar free/alcohol free mouth washes, lubricating gums, gels at night.

avoidance of exacerbating substances - Medications - antihistamines, antidepressants, other anticholinergics